MCID: ACQ016
MIFTS: 27

Acquired Pure Red Cell Aplasia

Categories: Blood diseases, Bone diseases, Immune diseases, Rare diseases

Aliases & Classifications for Acquired Pure Red Cell Aplasia

MalaCards integrated aliases for Acquired Pure Red Cell Aplasia:

Name: Acquired Pure Red Cell Aplasia 52 36
Adult Pure Red Cell Aplasia 52 58
Idiopathic Pure Red Cell Aplasia 52
Acquired Red Cell Aplasia 71
Acquired Prca 52

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

KEGG 36 H01586
ICD10 32 D60 D60.9
ICD10 via Orphanet 33 D60.0
Orphanet 58 ORPHA98872
UMLS 71 C0340961

Summaries for Acquired Pure Red Cell Aplasia

KEGG : 36 Pure red cell aplasia (PRCA) is a rare condition characterised by selective inhibition and the absence of red cell precursors in the bone marrow with consequent anaemia and reticulocytopenia in the presence of normal granulocytic and megakaryocytic lineages. RPCA can be classified into congenital and acquired. Congenital PRCA is also known as Diamond-Blackfan anemia [DS:H00237] and is a disease of infancy and early childhood. The aetiology of acquired PRCA can be subclassified into primary and secondary. Primary PRCA is defined when no aetiology can be identified using available investigations. The causes of secondary PRCA are again diverse and can be due to infections (viral, bacterial, etc.), drugs (erythropoietin, carbamazepine, etc.), collagen vascular disorders (rheumatoid arthritis, systemic lupus erythematosus, etc.), malignancies (e.g. thymoma), ABO-incompatible haematopoietic stem cell transplantation, and pregnancy. Depending on the cause, the course can be acute and self-limiting or chronic with rare spontaneous remissions. PRCA can be easily diagnosed when isolated anaemia, in the presence of normal white cell and platelet counts, is associated with a marrow of normal cellularity in which there is an almost complete absence of erythroblasts but normal myeloid cells and megakaryocytes. Primary, or secondary PRCA not responding to treatment of the underlying diseases, is treated as an immunologically-mediated disease. The therapeutic plan usually focuses on the sequential use of various immunosuppressive therapies until a remission is obtained.

MalaCards based summary : Acquired Pure Red Cell Aplasia, also known as adult pure red cell aplasia, is related to transient erythroblastopenia of childhood and red cell aplasia. The drugs Tacrolimus and Clotrimazole have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, bone and myeloid.

NIH Rare Diseases : 52 Acquired pure red cell aplasia (PRCA) is a bone marrow disorder characterized by a reduction of red blood cells (erythrocytes) produced by the bone marrow. Signs and symptoms may include fatigue, lethargy, and/or abnormal paleness of the skin (pallor) due to the anemia the caused by the disorder. In most cases, the cause of acquired PRCA is unknown (idiopathic). In other cases it may occur secondary to autoimmune disorders , tumors of the thymus gland (thymomas ), hematologic cancers , solid tumors, viral infections, or certain drugs. Treatment depends on the cause of the condition (if known) but often includes transfusions for individuals who are severely anemic and have cardiorespiratory failure.

Related Diseases for Acquired Pure Red Cell Aplasia

Diseases in the Pure Red-Cell Aplasia family:

Acquired Pure Red Cell Aplasia

Diseases related to Acquired Pure Red Cell Aplasia via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 46)
# Related Disease Score Top Affiliating Genes
1 transient erythroblastopenia of childhood 12.3
2 red cell aplasia 11.8
3 pure red-cell aplasia 11.0
4 thymoma, familial 10.5
5 thymoma 10.5
6 chronic kidney disease 10.3
7 autoimmune disease 10.2
8 leukemia, chronic lymphocytic 10.2
9 thrombocytopenia 10.2
10 acquired amegakaryocytic thrombocytopenia 10.2
11 systemic lupus erythematosus 10.1
12 anemia, x-linked, with or without neutropenia and/or platelet abnormalities 10.1
13 b-cell expansion with nfkb and t-cell anergy 10.1
14 deficiency anemia 10.1
15 thrombocytopenia due to platelet alloimmunization 10.1
16 lupus erythematosus 10.1
17 spondyloarthropathy 1 10.1
18 rheumatoid arthritis 10.1
19 myelofibrosis 10.1
20 coronary heart disease 1 10.1
21 graft-versus-host disease 10.1
22 immunodeficiency, common variable, 8, with autoimmunity 10.1
23 pulmonary aspergilloma 10.1
24 inflammatory bowel disease 10.1
25 t-cell large granular lymphocyte leukemia 10.1
26 follicular lymphoma 10.1
27 lymphoma 10.1
28 mucositis 10.1
29 chronic myelomonocytic leukemia 10.1
30 inflammatory spondylopathy 10.1
31 neutropenia 10.1
32 leukemia 10.1
33 hypothyroidism 10.1
34 purpura 10.1
35 kidney disease 10.1
36 connective tissue disease 10.1
37 spondylitis 10.1
38 b-cell lymphoma 10.1
39 end stage renal disease 10.1
40 herpes zoster 10.1
41 collagen disease 10.1
42 evans' syndrome 10.1
43 acute myeloid leukemia with abnormal bone marrow eosinophils inv(16)(p13q22) or t(16;16)(p13;q22) 10.1
44 splenomegaly 10.1
45 large granular lymphocyte leukemia 10.1
46 thyroid carcinoma 10.1

Graphical network of the top 20 diseases related to Acquired Pure Red Cell Aplasia:



Diseases related to Acquired Pure Red Cell Aplasia

Symptoms & Phenotypes for Acquired Pure Red Cell Aplasia

Drugs & Therapeutics for Acquired Pure Red Cell Aplasia

Drugs for Acquired Pure Red Cell Aplasia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 21)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tacrolimus Approved, Investigational Phase 4 104987-11-3 445643 439492 6473866
2
Clotrimazole Approved, Vet_approved Phase 4 23593-75-1 2812
3
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
4
Sirolimus Approved, Investigational Phase 4 53123-88-9 5284616 6436030 46835353
5 Immunosuppressive Agents Phase 4
6 Calcineurin Inhibitors Phase 4
7 Immunologic Factors Phase 4
8 Anti-Infective Agents Phase 4
9 Antifungal Agents Phase 4
10 Anti-Bacterial Agents Phase 4
11 Antibiotics, Antitubercular Phase 4
12
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
13
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
14
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
15 Hormones Phase 2
16 Antineoplastic Agents, Hormonal Phase 2
17 Hormone Antagonists Phase 2
18 Antiemetics Phase 2
19 Gastrointestinal Agents Phase 2
20 glucocorticoids Phase 2
21 Anti-Inflammatory Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Tacrolimus Treatment for Refractory Pure Red Cell Aplasia, a Prospective Study Unknown status NCT03540472 Phase 4 tacrolimus
2 Sirolimus Treatment for Refractory Pure Red Cell Aplasia, a Prospective Study Unknown status NCT03364764 Phase 4 Sirolimus
3 Safety and Efficacy of Bortezomib Plus Dexamethasone for Acquired Pure Red Cell Aplasia Failure or Relapse After First-line Treatment: A Prospective Phase II Trial Not yet recruiting NCT04423367 Phase 2 bortezomib/dexamethasone
4 Screening Protocol and Longitudinal Study of Bone Marrow Failure Syndromes and Cytopenias Unknown status NCT00315419

Search NIH Clinical Center for Acquired Pure Red Cell Aplasia

Genetic Tests for Acquired Pure Red Cell Aplasia

Anatomical Context for Acquired Pure Red Cell Aplasia

MalaCards organs/tissues related to Acquired Pure Red Cell Aplasia:

40
Bone Marrow, Bone, Myeloid, Thymus, Skin, T Cells, B Cells

Publications for Acquired Pure Red Cell Aplasia

Articles related to Acquired Pure Red Cell Aplasia:

(show top 50) (show all 122)
# Title Authors PMID Year
1
Pure Red Cell Aplasia and Antibody-Mediated Rejection: Double Trouble in 1 Kidney Transplant Recipient Solved by Intravenous Immunoglobulin Infusion: A Case Report. 61
32276841 2020
2
Immunosuppressive therapy for elderly-acquired pure red cell aplasia: cyclosporine A may be more effective. 61
31970447 2020
3
[Chinese expert consensus on the diagnosis and treatment of acquired pure red cell aplasia (2020)]. 61
32311886 2020
4
Acquired Pure Red Cell Aplasia Following Recombinant Erythropoietin (Darbepoetin-alfa) Therapy. 61
32269436 2020
5
Isoniazid Induced Acquired Pure Red Cell Aplasia: A Rare Case Report. 61
31979924 2020
6
Acquired Pure Red Cell Aplasia. 61
31979930 2020
7
Acquired Pure Red Cell Aplasia and Acquired Amegakaryocytic Thrombocytopenia Associated With Clonal Expansion of T-Cell Large Granular Lymphocytes in a Patient With Lipopolysaccharide-responsive Beige-like Anchor (LRBA) Protein Deficiency. 61
30188351 2019
8
Allogeneic stem cell transplantation for acquired pure red cell aplasia. 61
31396977 2019
9
Induced complete remission faster in adult patients with acquired pure red cell aplasia by combining cyclosporine A with corticosteroids. 61
31593095 2019
10
Acquired pure red cell aplasia in a patient with ankylosing spondylitis- a case report and literature review. 61
31277118 2019
11
Successful treatment of refractory/relapsed acquired pure red cell aplasia with sirolimus. 61
29982851 2018
12
The clinical characteristics and therapy response of patients with acquired pure red cell aplasia. 61
29754581 2018
13
Frequent STAT3 mutations in CD8+ T cells from patients with pure red cell aplasia. 61
30337298 2018
14
Response to cyclosporine A and corticosteroids in adult patients with acquired pure red cell aplasia: serial experience at a single center. 61
29589280 2018
15
Identification of mutations in patients with acquired pure red cell aplasia. 61
29767669 2018
16
Rational management approach to pure red cell aplasia. 61
29217782 2018
17
Successful treatment of tacrolimus-related pure red cell aplasia and autoimmune hemolytic anemia with rituximab in a pediatric cardiac transplant patient. 61
28598573 2017
18
[Clinical analysis of 32 patients with acquired pure red cell aplasia]. 61
29224325 2017
19
Successful high-dose dexamethasone treatment of acquired pure red cell aplasia following ABO major mismatched allogeneic hematopoietic stem cell transplantation. 61
27384857 2016
20
Cyclosporine restores hematopoietic function by compensating for decreased Tregs in patients with pure red cell aplasia and acquired aplastic anemia. 61
26968551 2016
21
[Acquired pure red cell aplasia in a patient with B cell chronic lymphocytic leukemia after fludarabine therapy]. 61
26256883 2015
22
[Chinese expert consensus on the treatment and diagnosis of acquired pure red cell aplasia (2015)]. 61
26031519 2015
23
[Integration of Internal and Clinical Laboratory Medicine]. 61
26524863 2015
24
Acquired pure red cell aplasia in a patient of rheumatoid arthritis. 61
25332592 2014
25
[Clinical assessment of CD4/8 ratio of bone marrow lymphocytes in acquired pure red cell aplasia]. 61
24975341 2014
26
Recurrent severe anaemia: a rare presentation of parvovirus b19 infection. 61
24959472 2014
27
Alemtuzumab plus cyclosporine treatment of the autoimmune hemolytic anemia in an adult bowel transplant. 61
25177510 2014
28
Acquired pure red cell aplasia due to treatment with clopidogrel: first case report. 61
24297264 2014
29
Low-dose subcutaneous alemtuzumab is a safe and effective treatment for chronic acquired pure red cell aplasia. 61
24310664 2013
30
Successful treatment of acquired pure red cell aplasia with oral corticosteroids in a patient with B-cell CLL. 61
24234432 2013
31
Acquired pure red cell aplasia due to anti-erythropoietin antibodies in a patient with end stage chronic kidney disease. 61
23817942 2013
32
[The pure red cell aplasia in children (observation 2010-2012 years)]. 61
23567308 2013
33
CDR3-independent expansion of Vδ1 T lymphocytes in acquired chronic pure red cell aplasia. 61
23274961 2013
34
Treatment of pure red-cell aplasia with cyclosporine in a renal transplant patient. 61
22891934 2013
35
Acquired Pure Red Cell Aplasia caused by Parvovirus B19 Infection following a Renal Transplant. 61
21969904 2011
36
Pure red cell aplasia caused by parvovirus B19 in two patients without chronic hemolysis. 61
20820839 2011
37
[Myelodysplastic syndrome with erythroblastopenia]. 61
20801559 2011
38
First case report of acquired pure red cell aplasia associated with micafungin. 61
21532231 2011
39
Pure red-cell aplasia as the presenting feature of the carcionoid tumor of the thymus: case report. 61
19224408 2010
40
Immunosuppressive treatment in patient with pure red cell aplasia associated with chronic myelomonocytic leukemia: harm or benefit? 61
19760482 2009
41
Pure red cell aplasia as first manifestation of splenic marginal zone lymphoma-successful treatment with rituximab: a case report. 61
19918496 2009
42
Diagnosis and management of acquired pure red cell aplasia. 61
19327582 2009
43
Acquired pure red cell aplasia associated with malignant lymphomas: a nationwide cohort study in Japan for the PRCA Collaborative Study Group. 61
19195037 2009
44
Rituximab to treat chronic lymphoproliferative disorder-associated pure red cell aplasia. 61
19067738 2009
45
Acquired pure red cell aplasia: updated review of treatment. 61
18510682 2008
46
[The clinical characteristics of T cell large granular lymphocyte leukemia associated with pure red cell aplasia]. 61
18844068 2008
47
[Aplastic anemia and pure red cell aplasia]. 61
18326319 2008
48
Three episodes of acquired pure red cell aplasia restricted to pregnancy. 61
18576941 2008
49
Primary Sjögren syndrome complicated by autoimmune hemolytic anemia and pure red cell aplasia. 61
18091373 2007
50
Antibody-mediated acquired pure red cell aplasia (PRCA) after treatment with darbepoetin. 61
17314208 2007

Variations for Acquired Pure Red Cell Aplasia

Expression for Acquired Pure Red Cell Aplasia

Search GEO for disease gene expression data for Acquired Pure Red Cell Aplasia.

Pathways for Acquired Pure Red Cell Aplasia

GO Terms for Acquired Pure Red Cell Aplasia

Sources for Acquired Pure Red Cell Aplasia

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68 SNOMED-CT via HPO
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70 Tocris
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72 UMLS via Orphanet
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